Handok said Monday that it has signed an agreement with Technoclone, an Austrian company, to supply the latter’s diagnostic reagent for a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS-13) activity test.

The ADAMTS-13 diagnostic reagent, which can confirm the test results within a few hours, is the first diagnostic reagent for enzyme immunoassay method test that has received approval from the Ministry of Food and Drug Safety.

The ADAMTS-13 activity test is a mandatory test for the differential diagnosis of the atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). TTP and aHUS are rare, life-threatening diseases, which require rapid diagnosis and treatment. However, the two conditions are too similar to distinguish, and the diagnosis of ADAMTS-13 enzyme deficiency is the only method to diagnose the disease.

In particular, aHUS may cause death or end-stage renal disease if the patient does not receive the right diagnosis and treatment. Studies have shown that up to 79 percent of patients have permanent renal damage, require dialysis, or die within three years of diagnosis.

Also, thrombosis and inflammation can cause thrombotic microangiopathy, which damages small blood vessels throughout the body, leading to damages in the major organs such as kidneys, heart, and brain, and various life-threatening diseases such as acute kidney failure and stroke.

“The number of patients with rare diseases is so small that it can take months to get the test results, which can lead patients to miss out on treatment,” Handok CEO Kim Young-jin said. “Since it is important for even one patient to be treated, the company has decided to supply the therapeutic agents as well as diagnostic reagents in Korea.”